Constant involvement of the Betz cells and pyramidal tract in amyotrophic lateral sclerosis with dementia: a clinicopathological study of eight autopsy cases.

We investigated clinicopathologically pyramidal signs, including hyperreflexia, Babinski sign, and spasticity, and the involvement of the primary motor cortex and pyramidal tract, in eight Japanese autopsy cases of amyotrophic lateral sclerosis (ALS) with dementia. Pyramidal signs were observed in seven (88%) of the eight autopsy cases. Hyperreflexia and Babinski sign were evident in seven (88%) and three (38%) patients, respectively, but spasticity was not observed in any of the eight patients. Loss of Betz cells in the primary motor cortex was evident in the seven cases in which this structure was examined. Astrocytosis in the fifth layer of the primary motor cortex was noticed in three cases. In all eight cases, involvement of the pyramidal tract was obvious in the medulla oblongata, but no involvement of the pyramidal tract was found in the midbrain. Involvement of the pyramidal tract in the spinal cord, particularly of large myelinated fibers, was observed in all six cases in which the spinal cord was examined. In ALS with dementia, pyramidal signs were shown to be present more frequently than previously believed, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells and the pyramidal tract in ALS with dementia has not been reported. Our clinicopathological findings may make a contribution to the understanding of the clinicopathological hallmarks of this disorder. Furthermore, we believe that this study will also contribute to the elucidation of the nosological status of ALS with dementia.

Effects of pulsed ultrasound on development and glucose uptake of preimplantation mouse embryos.

The effects of pulsed ultrasound (US) on early mouse embryos were investigated. Two-cell embryos contained in oviducts were irradiated to US (1.875 MHz with an I (SPTA) of 2.96 W/cm(2)) in an experimental unit for either 1 or 5 min (exposure groups). The embryos were cultured to examine the rate of developing to blastocysts, and the uptake of 2-deoxyglucose (2-DG) into blastocysts was measured to evaluate their viability. The rates in the exposure groups were essentially the same, with those of the embryos treated similarly in the unit unless being exposed to US (nonexposure groups). However, they were lower than that of embryos not treated in the experimental unit (a control group). There were no significant differences of 2-DG uptake among the 1-min exposure, 1-min nonexposure, and control groups. The uptake in the 5-min exposure group did not differ from that in the 5-min nonexposure group; however, uptake in both groups was lower than that in the control group. Pulsed US for 1 min did not affect viability of preimplantation mouse embryos.

Effect of high amniotic fluid pressure on fetal circulation.

The purpose of this study was to investigate the effects of high amniotic fluid pressure (AFP) created by amnioinfusion on fetal circulation. Five pregnant goats whose fetuses had a mean gestational age of 131 +/- 2 days (term 145 days) were used. After a 30 minute control period, 1500 ml of warm saline was infused to the amniotic cavity over a period of 30 minutes. The AFP gradually increased during amnioinfusion and remained 2-3 mmHg above the control level for 3 hours after amnioinfusion. Fetal arterial pH (FpH) tended to decrease, and there was a significant negative correlation between the FpH and AFP (r = -0.723). Fetal arterial pO2 also tended to decrease and negatively correlated to the AFP (r = -0.59). On the other hand, fetal arterial pCO2 tended to increase and positively correlated with the AFP (r = 0.75). Although amnioinfusion is useful for improving the status of a distressed fetus, the fetal condition may be jeopardized at the same time. Therefore, careful monitoring of the AFP and fetal status is recommended during amnioinfusion.

The cerebellar and thalamic degeneration in Fukuyama-type congenital muscular dystrophy.

We report a male autopsy case of Fukuyama-type congenital muscular dystrophy (FCMD), with unusual neuropathological findings. The patient was a Japanese man aged 26 years at the time of death. He had shown severe psychomotor retardation and muscular dystrophy since early infancy, and was diagnosed as having FCMD at the age of 5 years. He died of respiratory failure. The main neuropathological finding was extensive cerebral and cerebellar cortical dysplasia, characteristic of this disorder. In addition, degeneration of the cerebellar efferent pathway, including the dentate nucleus, superior cerebellar peduncle, and red nucleus, and that of the lateral thalamic nucleus were observed. These findings suggest the possibility that the long survival can clarify the latent neurodegeneration in the cerebellum and thalamus in FCMD, in addition to congenital malformations. The system degeneration should be carefully evaluated in the pathological examination of this disorder.

Differential effect of exogenous human chorionic gonadotrophin on progesterone production from normal or malfunctioning corpus luteum.

To examine whether luteal phase defect is, in part, causally related to insufficient gonadotrophin stimulation, we compared the relation of the increment of serum progesterone concentrations in response to human chorionic gonadotrophin (HCG) with its basal level at mid-luteal phase. Thirty-eight naturally cycling infertile women aged between 27-41 years old were evaluated for hormonal responses to HCG injection at the mid-luteal phase. We measured luteinizing hormone (LH), follicle stimulating hormone (FSH), oestradiol and progesterone concentrations, before and 1, 2 and 3 h after the administration of HCG (5000 IU, i.m.) 7 days after ovulation verified by ultrasonography. Eleven out of 38 women exhibited progesterone concentrations below 10 ng/ml (low progesterone group), and those remaining showed progesterone concentrations of > or = 10 ng/ml (normal progesterone group). The basal LH, FSH and oestradiol concentrations were essentially the same in both groups. Progesterone concentrations rose significantly 1 h after the injection and levelled off thereafter. The increment of progesterone concentrations at 1 h in the normal progesterone group was 5.7 ng/ml on the average, whereas that in low progesterone group was 1.1 ng/ml. Furthermore, the percentage increase in progesterone concentrations at 1 h in the normal progesterone group was significantly greater than that in the low progesterone group. Both groups equally exhibited significant but marginal increases in oestradiol concentrations 1 h after the injection. LH and FSH concentrations at 3 h decreased significantly in both groups. In summary, HCG readily stimulates progesterone production in normally functioning corpus luteum whereas its stimulatory effect is minimal on malfunctioning corpus luteum. This suggests that luteal phase defect is not caused by inadequate gonadotrophin stimulation and, therefore, does not benefit from HCG administration.

Distribution of cerebellar cortical lesions in multiple system atrophy: a topographic neuropathological study of three autopsy cases in Japan.

We investigated neuropathologically the distribution of the cerebellar cortical lesions in three Japanese autopsy cases of multiple system atrophy (MSA) using hemisphere specimens. The lesions were classified as mild, moderate or severe. The distribution of cerebellar cortical lesions in all three cases were uniform: the cerebellar cortical lesions were more conspicuous in the vermis than in the hemisphere. These neuropathological findings differ from the established theory that cerebellar lesions of MSA are more pronounced in the hemisphere than in the vermis. The degree of cerebellar cortical lesions in our cases increased in relation to the duration of the disease. Our pathological data may contribute to the morphological differential diagnosis in various neurodegenerative disorders including late cortical cerebellar atrophy. Our neuropathological findings may also make a contribution to the neuroradiological progress in the differential diagnosis of spinocerebellar disease.

Fetal circulatory responses during maternal bleeding.

The purpose of this study was to investigate the fetal circulatory responses during maternal hemorrhage. Five pregnant goats with fetuses with a mean gestational age of 132 +/- 2 days were used. The maternal blood was withdrawn at 350 ml/h for 2 hours (e.g. 700 ml, 11.9 ml/kg maternal weight) and reinfused at the same speed. During maternal bleeding, maternal arterial pressure (MAP) gradually decreased. As a result of this maternal hypotension, fetal arterial pO2 and pH decreased, and PCO2 increased. After the reinfusion, fetal pO2 recovered but pH and PCO2 did not recover. Fetal arterial pressure (FAP) increased and heart rate (FHR) decreased during maternal bleeding and returned to the control level, after the reinfusion. Fetal arginine vasopressin (AVP) concentration increased to 401.2 +/- 318.5 pg/ml at the maximum bleeding. There were significant positive correlation between AVP concentration and FAP, and negative correlation between AVP and FHR during maternal bleeding. Therefore, we concluded that 700 ml maternal bleeding for 2 hours resulted the decrease in fetal pH, pO2, and FHR, and increase in PCO2, FAP, and AVP concentration. Fetal pH, PCO2, and AVP did not return to the control level in spite of reinfusion.

[Comparison of fetal circulatory responses during maternal bleeding and fetal bleeding].

The purpose of this study was to compare the fetal circulatory responses during maternal hemorrhage and fetal hemorrhage. Four pregnant goats, gestational age 131 +/- 7 days for the fetal hemorrhage and 5 goats, 132 +/- 2 days (term 145 days) for the maternal hemorrhage were used. The amounts of hemorrhage were 700ml per 2 hours for the maternal hemorrhage and 40ml per 2 hours for the fetal hemorrhage. Although fetal arterial pH decreased during both hemorrhages, fetal arterial pO2 increased during the fetal hemorrhage and decreased in the maternal hemorrhage. The fetal arginine vasopressin concentration and plasma renin activity increased during both hemorrhages, but the rates of change in the hormone concentrations were higher during the fetal hemorrhage. The fetal aldosterone concentration decreased during maternal hemorrhage but increased during fetal hemorrhage. FAP increased and FHR decreased during maternal hemorrhage. FAP and FHR were significantly correlated to the AVP concentration. These relationships were not found during fetal hemorrhage. We therefore concluded that fetal responses were completely different in pO2, FAP and FHR during the maternal and fetal hemorrhages.

Pancreatic fibrosis in patients with chronic alcohol abuse: correlation with alcoholic pancreatitis.

OBJECTIVES: To investigate the incidence and histological features of pancreatic fibrosis, including chronic alcoholic pancreatitis, in patients with a history of chronic alcohol abuse. METHODS: Forty-six autopsy cases of alcoholic dependence syndrome, 53 cases of chronic alcoholism, and 30 cases of chronic alcoholic pancreatitis were studied histopathologically. RESULTS: Fibrosis was seen in 33 of 46 cases of alcoholic dependence syndrome, 20 of 53 cases of chronic alcoholism, and all 30 cases of chronic alcoholic pancreatitis. Fibrosis was categorized into three types: intralobular, perilobular, and mixed intralobular and perilobular sclerosis. In chronic alcoholic pancreatitis, fibrosis was found mainly in perilobular, or interlobular, areas, and in some advanced cases extended into intralobular areas, so that the pancreatic tissue was completely replaced by fibrosis. Hence, interlobular fibrosis was found in all cases of chronic alcoholic pancreatitis. In contrast, in cases that had predominantly intralobular fibrosis, which were usually cases of alcoholic dependence syndrome, the pancreatic tissue had not completely disappeared, even at an advanced stage, and some parenchymal regeneration similar to that seen in hemochromatosis was observed. CONCLUSION: Interlobular and intralobular pancreatic fibrosis associated with alcohol abuse appears in distinct pathological patterns with differing mechanisms.

[A 64-year-old man with recurrent blurred vision and an abdominal mass].

We report a 64-year-old man with recurrent bouts of blurred vision who died after developing an abdominal mass. He was well until June of 1985 when he was 59-years-old when he had an acute onset of loss of vision in his right eye. He was treated by prednisolone with a complete remission. In August of 1986, he had another bout of blurring of vision in his left eye. Once he lost his left vision completely, from which he showed slow recovery. In January of 1987, he developed blurring of his right eye and loss of pain and touch sensation in his right leg. Since then he repeated loss of vision in his right or left eye five times, and he was admitted to our hospital in May of 1990. On admission, he was alert and oriented. General physical examination was unremarkable. Neurologic examination revealed bilateral optic nerve atrophy. He could not discriminate light or dark by either eye. Other cranial nerves were unremarkable. He could walk in a wide-base only with support; spasticity was noted in his left leg. Muscle strength was preserved. Deep reflexes were exaggerated in both legs with extensor plantar reflex bilaterally. Pain and touch sensation was decreased in the left leg by 30%, and vibration was diminished in both feet. Position sense was preserved. Routine blood counts and chemistries were unremarkable. Cranial MRI scans revealed multiple high-signal intensity lesions in both pontine bases, basal ganglia, thalami, and in the deep cerebral white matters. He was treated with oral prednisolone, plasmapheresis, lymphocytapheresis, and then immuran. His vision showed only slight recovery to discriminate light and dark. In October of 1990, slight weakness appeared in his both legs. In December of that year, he developed nausea, and a fiber colonoscopic study revealed a stenosis in the transverse colon. In March of 1991, he developed anemia and liver dysfunction. In July of that year, jaundice appeared, and his serum bilirubin was increased. In October, his leg weakness became more prominent, and his cranial CT scans at that time revealed a low density change in the right cerebellum in the right superior cerebellar artery territory; in addition, multiple low density spots were scattered to be seen in both cerebral hemispheres including the basal ganglia and thalamic areas with ventricular dilatation and cortical atrophy.(ABSTRACT TRUNCATED AT 400 WORDS)

[Autopsy results of an elderly case of Parkinson's disease and aorto-esophageal fistula who died of a massive hematemesis during prolonged bed rest].

An 81-year-old female patient with an 8-year history of Parkinson's disease was hospitalized because of aspiration pneumonia. The clinical course of her pneumonia was prolonged because of dysphagia with a short period of remission, and she required a long period of bed rest. She received supportive nutrition via a nasogastric tube and many peroral medications that consisted of 3 anti-Parkinsonian drugs and 5 anti-bacterial or anti-tussive agents. Six months after admission, she vomited fresh blood through the nasogastric tube, then went into hypovolemic shock. Hemodynamic stability was temporarily achieved by blood transfusion. Gastroduodenal endoscopic examination could not reveal the exact bleeding site because of massive blood clots. Five days later, the patient died of a massive hematemesis. Autopsy revealed 2 chronic longitudinal ulcers, each 1.7 x 0.4 cm in size, in the upper portion of the esophagus. One of them had developed a fistula to the aorta. Neither esophageal carcinoma nor a foreign body was detected around the fistula. Atherosclerosis of the aorta was mild and the perforation channel was covered with the esophageal epithelium. The fistula was assumed to be a product of local esophageal injury due to drug retention.

[A 93-year-old woman presenting consciousness disturbance, left hemiplegia, and respiratory distress].

We present a 93-year old female who abruptly developed left hemiplegia on the way to the wash room. She was admitted to another hospital where her consciousness deteriorated and artificial respiration was necessary. She was transferred to our hospital 20 days after the onset of her stroke. On admission, her BP was 60/40 mmHg, and wheezing râle was heard in both lung fields. No heart murmur was present. She was in comatose state, but a small amount of spontaneous movements were present in her eyelids and the jaw. Neurologic examination revealed no papilledema, small non-reactive pupils with the right side being slightly larger than the left, absence of ocular movements, and loss of oculocephalic response and corneal reflex. An intratracheal tube was in situ, and the observation of the pharynx was impossible. No stiff neck was present. No spontaneous movement was observed in her limbs which were flaccid, and all the deep reflexes were lost except for jaw jerk which was weakly positive. Cranial CT scans revealed low density areas in the right anterior and middle cerebral artery territories with patchy areas of high density being consistent with a hemorrhagic infarction. In addition, a small high density mass lesion was noted in the left frontal lobe adjacent to the falx, which was thought to be a small meningioma. Her clinical course was complicated by pneumonia, hypotension and cardiac failure. She expired 2 months after the onset of her stroke. Postmortem examination revealed an extensive hemorrhagic infarction involving the territory of the right internal carotid artery.(ABSTRACT TRUNCATED AT 250 WORDS)

[Forty-six-year-old woman with progressive external ophthalmoplegia and limb weakness].

We report a 46-year-old female who presented progressive ophthalmoplegia and limb weakness. She was well until the age of 15 years when there was an onset of bilateral deafness. She became completely deaf by 20 years of age. She noted an onset of weakness in her legs when she was 27-years-old and of ptosis at 34 years of age. She was admitted to our hospital when she was 41-years-old. Neurological examination revealed near total ophthalmoplegia, bilateral ptosis, dysphagia, generalized muscle atrophy and weakness of approximately 4/5 degree, facial grimacing, athetotic movements in four limbs. Laboratory examinations revealed increase in blood lactate and pyruvate levels and diffuse low density change in the cerebral white matter in CT scans. She was thought to have a mitochondrial encephalomyopathy. She was discharged for follow-up, but her clinical course was that of a relentless deterioration. She was readmitted to our service in December 1989. She showed further progress in her weakness and muscle atrophy. Otherwise neurological examination was essentially similar to the previous one. Her cranial CT scans showed low density changes in striatum, thalamus and midbrain in addition to the white matter. Enzyme activities of the electron transport complexes revealed a moderate decrease in the succinatecytochrome c reductase activity, and the Southern blot analysis of mtDNA revealed multiple deletions in mitochondrial genomes. Two months after her admission, she developed bronchopneumonia, and expired on March 13th, 1990. Post-mortem examination revealed diffuse pallor of myeline in the cerebral white matter in K-B staining. A marked neuronal loss and gliosis were observed in putamen bilaterally. Skeletal muscles showed typical changes of mitochondrial myopathies with ragged-red fibers in Gomori-Trichrome staining, and crystalline inclusion bodies by electron microscopic observations. Some neurogenic atrophies were also seen. Oculomotor nuclei appeared intact. It was thought that she had an incomplete form of Kearns-Sayre syndrome. The patient was discussed in a neurological CPC of the departments of Neurology and Pathology of Juntendo University School of Medicine.

Necrotizing sialometaplasia in the mouth floor secondary to reconstructive surgery for tongue carcinoma.

Necrotizing sialometaplasia is a benign inflammatory process, which histologically can mimic squamous cell carcinoma. A 63-year-old man underwent left hemiglossectomy involving transplantation of a myocutaneous flap for squamous cell carcinoma of the tongue. One month after the operation, necrotizing sialometaplasia occurred in the minor salivary gland tissue of the mouth floor, compressed by the necrotic flap. This case is very unusual because of the occurrence of necrotizing sialometaplasia in the floor of the mouth. The etiology of the lesion was considered to be ischemia secondary to compression by the necrotic myocutaneous flap.

Osteogenic sarcoma of the tibia in a patient with epidermolysis bullosa dystrophica.

An osteogenic sarcoma of the tibia occurred in an eight-year-old boy with epidermolysis bullosa dystrophica hereditaria (EBDH) (Hallopeau-Siemens type). The patient had had the congenital skin disease since the time of birth. A painful swelling appeared in the proximal portion of his right tibia, which was diagnosed as osteogenic sarcoma on biopsy study. The patient died of massive pulmonary metastases and cachexia four months after the onset of the tumor. Osteogenic sarcoma in a patient with EBDH seems not to have been previously reported. EBDH has been known to be occasionally associated with squamous cell carcinoma. Whether an impairment of the patient's defense mechanism by the chronic skin disease might have enhanced the rapid progression of the associated bone malignancy is unknown.

Counter measures against death from arrhythmias in acute myocardial infarction: mortality of the disease in a hospital at different periods with changing care systems. Factors of delay in the pre-hospital phase.

The results of treatment of 400 patients with established acute myocardial infarction in a hospital at different periods with changing care systems are described. Significant reduction of death from arrhythmias could be obtained only when an organization with immediate availability of well-trained medical personnel and reliable actual continuous monitoring of electrocardiograms had been established. Components of delay in the pre-hospital phase of acute myocardial infarction were also studied. There were two significant factors in delay: one was delay due to ignorance of patients about the disease, second was the delay after consultation of family doctors until arrival at the CCU. Time is consumed in the latter factor mainly in making of definite diagnosis. The implication of these data in the management of patients with a diagnosis of acute myocardial infarction are discussed.

Unusual mechanism in the initiation of the paroxysmal supraventricular tachycardia in a patient with WPW syndrome.

A heretofore unreported unusual mechanism in the initiation of the paroxysmal supraventricular tachycardia (PSVT) in a patient with WPW (Wolff-Parkinson-White) syndrome was observed using His bundle recordings. The patient initially had some degree of AV conduction disturbance at the level of the AV node. A premature atrial impulse initially activated the ventricle exclusively through the accessory pathway and the same impulse re-excited the ventricle via the AV nodal-His axis after finishing the pure pre-excitation with marked prolongation of the AH and the HV intervals. After finishing this double ventricular response it traversed to the atrium to produce the PSVT.